Contents

Penelope's Gossip Corner
Dates for your diary
Letter from America by Lynn Erstein
Information for Carers by Carol Jennings
My Hero, My Dad by Rachel Beaumont
Young Persons Day by Carol Jennings
Our Son Mark by Margaret and Neville Haddock
Obtaining a Diagnosis by Niki Philcox
Sex Sex & No Sex by Penelope Roques
Looking Ahead: viewpoint from Philadelphia
Information Sheets
Contact Details
PDSG E-Mail Directory

PENELOPE’S GOSSIP CORNER

CANDID held another study day in the 18th and the group (Kate did the organising) had arranged a festschrift for me after that. For two hours they discussed the work I had been involved in and managed to highlight some of my managerial skills (apparently I used to start meetings with Martin Rossor by telling him to "sit down and concentrate", at least that's what he says). Angus Kennedy ran a This is Your Life session with photos of me (I am still not talking to my mother) and finished with a slide which he says was a copy of the PET scan I had as a volunteer (a most attractive brain). Our Chairman David Hunter spoke, as did Carol Jennings and Clive Ever of the ADS. All enormous fun and all my favourite people came. It was amazing to look up in the lecture theatre and see it full of friends wishing me well.

We had PDSG meetings in London and Liverpool. Martin Gillan from Alzheimer Scotland Action on Dementia came to the London Meeting and we hope to hear from Martin about meetings being held in Scotland. Fiona Johnson came to the Liverpool meeting. Fiona is a nurse who has a special responsibility for younger sufferers and their carers. She will try to come to our Liverpool meetings. Fiona can be contacted at the Glaxo Neurological Centre on 9/7/98, 13/8/98, 10/9/98, 8/10/98, 12/11/98 and 10/12/98. If you want advice I warmly suggest you contact her on those dates, telephone number 0151 298 2999. The hospitality at the Glaxo Neurological Centre was, as always, great.

There have been several encouraging reports of identification of a faulty gene causing Frontal Lobe Dementia in families with the hereditary form of the illness. We will be discussing these in more detail in the next Newsletter.

The PDSG has been represented at several meetings recently by David Hunter, Carol Jennings or myself. The next meeting is at Nottingham on 3rd October. The British Lions. Leicester have very kindly given the PDSG a printer and I am setting up the PDSG at my home. I am sorry if E-Mails and letters don't get answered at first but I have been busy with holidays and organising the transition (piles of papers at home instead of at work) but our PDSG usual efficiency will resume soon.

We enjoyed ourselves at Kew Gardens but haven't really got the designated picnic area sorted out. We will have perfected it by next year (by the way the sun shone).

In Memory

Please could we remember Christopher Thompson.

DATES FOR YOUR DIARY

We would very much like to organise a day excursion to France to meet the Fronto-temporal Group in Lille. This will be next year for carers and sufferers. Please will anyone interested in coming along contact me and I would especially like to hear from people interested in helping me organise the day.

Letter from America by Lynn Erstein

"Sit down before your read this, as I must pass on to you some not so good news. Up front, your mother has a condition that relates to a slow deterioration. The neurologist says that it’s treatable but not curable. Science has not figured out yet how this comes about nor can they predict the rate of deterioration. Although it’s not good news, it does explain the problems we have all noted. The fancy medical name is Alzheimer’s disease, named after the German doctor who first described it in 1906".

This was written almost seventeen years ago, and although Alzheimer’s is now a household word, and doctors know much more about its molecular pathology, science has still not figured out how this comes about nor can they predict the rate of deterioration. We now had a reason for the strange things we had been noticing during the last few months:

Speech and Language

One of the first signs we noticed was that my mother would repeat herself, asking the same question over and over and retelling old stories. The sensory aphasia had surprised us, but we had been ignoring this misuse of words for some time. She had been a very articulate woman, so it especially pained us later to have to help her figure out what she wanted to say when she experienced motor aphasia. Her muscles just wouldn’t move to pronounce the words she wanted to say. The slowed and slurred speech was startling because proper enunciation had always been so important to her. There were paraphrasic errors that we later learned were important indicators of aphasic language disorder. She would substitute first just a word and then later strings of words that made understanding what she was trying to say an ever increasing challenge. As she was losing expressive language, her sentences got shorter and shorter. Finally she would give up speaking altogether but continued to communicate by writing for almost another year.

Judgement-both logical and social capacities

She had been a very good, careful driver but we had noticed some disturbing lapses in judgement, such as driving too fast. Her visual agnosia also made driving dangerous as she lost her depth perception and ability to understand what a stop sign even meant. As a diplomat’s wife, she had all the proper social graces, but now she was interrupting people with totally different topics, hanging up the phone mid-conversation, and later would drink from other people’s glasses at the dinner table, not know when to stop eating to the point of vomiting and just have trouble functioning in a social situation. This was especially sad as she wanted so much to continue her old relationships with friends but it became increasingly difficult.

Memory

This was first noticed when travelling. She had trouble remembering new information like where the car was parked. Although at the beginning only short term memory was affected, later we would have to deal with problems like her forgetting how to turn off the bathtub faucet and the stove burners.

Ability to Abstract

Her thoughts as seen in her diminished conversation were becoming more shallow. Her ability and enthusiasm to discuss world events and history was leaving.

Emotional Responsiveness

My mother, a usually outgoing person, was becoming more quiet and aloof. As previously her face and voice would be animated in conversation, she would eventually have a flat affect.

A short time later I read about Alzheimer’s Disease. The article also went on to tell about a related condition, called Pick’s disease or Pick’s convolutional atrophy with similar symptoms plus atrophy of the temporal and frontal lobes of the brain. To arrive at the diagnosis, my parents had made the rounds of doctors, internists, neurologists, and psychologists. Since there were no deductive tests available we had to rely on inductive testing. Her regular internist did the physical exam including a complete blood and urine work-up to rule out vascular, infectious and metabolic disorders, hepatic encephalopathy, certain lung diseases and depression. She was put on Ritalin to combat any depression and help with distractibility. From the mental status testing, the psychiatrist doubted she was suffering from a psychiatric syndrome and specifically doubted that depression was a likely cause of her clinical problems.

My mother was fortunate to be admitted to the Alzheimer’s research program at the National Institutes of Health, in the National Institute of Neurology, Communicative Diseases and Strokes, near Washington DC. Her neurologist there primarily did research and had only 32 patients in his program. The program was free to us. They would identify her problem, monitor her twice a year, perform the latest tests and make recommendations to be carried out by her regular physician. If there were any breakthroughs she would be among the first to have them available. In return, she would be studied to benefit Alzheimer’s research and to expand knowledge of the disease. After her death they would perform a full autopsy and use her brain to further the study of dementia. A thorough work-up during a three week hospital stay at NIH, revealed everything to be within the normal range except the CT scan of the head, which revealed diffuse sulci widening with hydrocephalus ex-vacuo. It showed prominent dilated ventricles, prominent subarachnoid spaces and cortical sulci, most likely representing brain atrophy. The report from the physician in charge stated, "Overall my impression is that the patient is suffering from a disorder which has resulted in a personality change over the course of the past year with some memory, language and apraxic difficulties. There is insufficient evidence to attribute this disorder to a psychiatric problem and it seems entirely possible that the patient is actually suffering from Alzheimer’s disease which is presenting in a very unusual fashion and could, in fact be Pick’s disease".

Personality, behaviour and orientation changes tend to come before memory loss in Pick’s disease, unlike Alzheimer’s disease. From a PET scan, taken less than a year after the first diagnosis, it was apparent that the major involvement was in the right frontal lobe- the area that controls personality, speech, and behaviour. Even my father, with no medical background, could see from the scans, that the right hemisphere of the brain was diminished.

In 1983, one of the leading theories was that Alzheimer’s disease. was due to an imbalance in the chemistry of the spinal cord fluid. A drug was approved and tried based on the fact that the brain works by transmitting small electrical charges through fluid from neuron to neuron. Starting in September 1983, she spent 6 weeks in NIH to monitor the side effects from a new drug, THIP. While there she took part in a seminar on dementias for 50-75 doctors. The drug did not help and later trials were given to patients who were in earlier stages of the disease. During the seven years my mother was in the nursing home, she continued to be monitored semi-annually at NIH. She died of pneumonia on November 21, 1992.

Autopsy Report

A complete autopsy was given at 9:30 AM on November 22, 1992. The part of the report covering the brain-stated that the cerebral hemispheres were symmetrical with marked circumscribed frontotemporal atrophy . Sections of the grossly abnormal areas (frontal and temporal cortex, and to a lesser extent head of caudate), reveal marked cell loss and gliosis in the grey matter. Numerous amphophilic intraneuronal inclusions (Pick bodies) are present. Some ballooned neurons are also seen. The neuropathological findings are typical of Pick s disease, and well explain the clinical findings. It is of interest to note that Pick bodies, which are absent in up to 2/3 of cases of circumscribed atrophy, are numerous here. Although there was no definitive test for Pick’s disease, it turned out that some of the early theories were correct.

The pain and frustration of watching my mother drift away from us was devastating. The only good to come of it is knowing that perhaps she helped scientists understand the disease which will eventually lead to a cure.

INFORMATION FOR CARERS

We can assume that it would be useful to have an A5 booklet containing all the information included in the fact sheets, plus some of the excellent articles from the newsletter.

EUREKA!! The Leicester Lions Club wanted to support our work in some way and agreed to sponsor such a booklet. With their help and the expertise and generosity of Phoenix Photo Litho printers this booklet will be available in the next couple of weeks.

Its glossy cover proclaims 'Pick's Disease Support Group - INFORMATION FOR CARERS' and it has sections including symptoms of some of the diseases we cover, problems encountered such as eating and money and essential information - for example legal and financial matters.

We hope it will be useful to new and more experienced carers alike.

Do let me know if you would like a copy.

Carol Jennings

My Hero, My Dad
Rachel Beaumont

I think Dad was a hero for being amazingly brave.

YOUNG PERSONS DAY

We would like to run a young persons day. This will be very informal - in fact we are planning on descending on Penelope's (so far) peaceful home in Northwood, Middlesex!!

Parents and young people are invited to come along. We would like to lessen those feelings of isolation, talk through problems, discuss questions you may have and share ideas.

To make this viable we need at least two families. I have found it very difficult to make contact (summer holidays I'm sure have got in the way) and to find a date suitable for those concerned.

Now it's over to you. If you would like to be a part of this day please get in touch with me and give me an idea of dates that would be suitable - or at least dates that are out of the question.

We want this new venture to work and need your input. Come and tell us how we can best support YOU.

I look forward to hearing from you.

Carol Jennings

carol@jengs.force9.co.uk

OUR SON MARK

Mark was 37 years old yesterday so he was 31 years old when we began to realise that he was acting strangely but thought it maybe was stress at work. He was unsettled in his job, so came to work in our shop, but again couldn’t settle, so went back to his old firm.

Mark is married with two children. He was very keen on walking and youth hostelling. At the start of his illness his children were 5 and 6 years old and he thought the world of them; they were one of the happiest families I know.

He had what we realise now were the symptoms of Pick’s disease. Obsessive behaviour, and repeating over and over again what he had just said or done, talking to strangers in the street, going out for a walk, coming home and immediately going out again. It became very difficult to cope with his behaviour. His wife was trying to keep her part-time job going, (she’s an occupational therapist with the local authority) and cope with the children. We helped as much as we could, but we lived half an hours drive away from them. Anyway, a crisis eventually arose. Mark was beaten up while on one of his walks round the local reservoir and obviously had made no attempt to defend himself. After hospital treatment he was allowed home. So he was then given day care at home for a short while, but there were still the nights and weekends to cope with.

Eventually it was decided that he needed more permanent care and he went to a residential home in the next town, where he was for about 15 months, until they could no longer manage him. Mark was then admitted to the local hospital for assessment and a care plan to be worked out. Then he was placed in a community care home 5 minutes drive from his own home and about 20 minutes from our home, which was ideal.

His care there has been very goo. He was given one-to-one care all day and night with staff taking 30 minute turns to be with Mark as he was quite active at all times, only sleeping about 3 hours a night. Each resident in the home has a qualified nurse, an auxiliary nurse and a care worker assigned to them, and they in turn are responsible for 5 or 6 patients each.

Mark is on very little medication, just enough to stop him feeling stressed. In the first year there they would take him out to play football, swimming or walking, which was difficult as he needed 2 carers if he went out.

They were eventually allotted extra money for Mark, as he was a young man, in with much older people in various stages of dementia. This enabled them to take on 3 helpers for Mark, which of course meant he could go out more. His activities this year vary from swimming, walking, trampolining, hydrotherapy and using a snoezoleum room on a regular basis, to a five day holiday at Centre Parks in January (this had to involve 6 carers). When the weather has been good they have taken him to the seaside, the zoo and various parks. They have tried very hard to give him a good quality of life and one can only hope that Mark has derived some pleasure and stimulation from it all, as he can no longer show his feelings.

He doesn’t talk now, except for an odd repeated word. Eating and drinking are difficult for him, he can swallow but the food comes out many times before it goes down. Mealtimes can be rather messy, but he gets there in the end.

Now over this last month, he is much quieter, less energetic, sits down for much longer periods. He now only can walk for a short time before he obviously needs to sit down. His pulse rate has become much more erratic. On a night he seems content to stay in bed when awake, they have a "child’s listening device" in his room so whoever is in charge of him can hear if he decides to move. He has a stereo in his room and plenty of "mood" discs, which he is content to listen to.

We realise these changes are maybe deterioration in his illness, but know very little about what the future holds for Mark.

Over the years in care his wife and children have continued to visit him twice a week. The children, now 10 and 11 years, are very good with Mark. They all still love him very much and have coped wonderfully with his illness thanks to his wife who has told them about his illness from the beginning in a way for them to understand. She in fact has kept us going with her practical outlook and strength. We also visit Mark twice a week and are happy with the care he is receiving.

Last night we all visited Mark for his birthday. The staff had made a cake and a buffet tea and his wife brought in a cake, so we had two lots of candles to blow out and lots of Happy Birthdays to sing. At Christmas we went to the home for Christmas dinner which was very good.

Now with Mark we take each day as it comes.

Margaret and Neville Haddock.

Obtaining a Diagnosis

I personally think that a clear diagnosis helps, and I would go wherever necessary to get one that you feel comfortable with. Dealing with the disease and the patient is easier if you understand what behaviours are "normal" and what to expect.

Good luck!

Niki Philcox

A good description of Pick’s/FLD is that the wiring in the front part of the brain has gone haywire. Sometimes there is no message getting through at all and sometimes messages are bombarding and cannot be switched off. When this happens more rational signals of caution seem to fail.

In some cases a sufferer of Pick’s/FLD will have loss of libido and show no interest in sex or consideration for the partner’s feelings. This may lead to suddenly having separate bedrooms and leaving the partner feeling shut out and isolated. Suggestions from the partner that they too have needs are totally ignored and there is no room for negotiation. After a long and close partnership this can be very hurtful.

Sometimes when rejection happens later in the illness we may wonder if perhaps the sufferer fails to recognise the face of the partner who after all, in their mind, is not this fifty plus person but is only 24 and very good looking!

The carer may also be so tired by their role of caring that they may not want any intimacies and in any case their feelings for their partner may have changed. Often a carer feels that the person they are caring for is not the person they married and their feelings have altered. They still love the person for whom they are caring but not in a sexual way.

We often describe a sufferer of Pick’s/FLD as being selfish and childish and appearing not to consider other people’s views. This can also be apparent in a sexual relationship. Failure to consider the partner’s needs is often mentioned. Encouraging the partner and explaining needs can work in some instances, but by no means in all cases.

Sometimes in cases of Pick’s/FLD we hear of changes in personality, particularly with patients becoming disinhibited. This may start with crude jokes told at inappropriate times and inappropriate comments. Such patients may also show increased interest in mildly perverted magazines for example. Disinhibition may also occur in the physical sense such as touching a partner inappropriately, which may cause great distress, particularly added to all the other stresses caused by Pick’s/FLD.

Pick’s/FLD causes immense sadness and the loss of small intimacies such as holding hands or the little pat on the shoulder can add to the sense of isolation and loneliness that these illnesses bring. Obtaining a diagnosis may provide some comfort. It is often only with hindsight that a carer can see when the onset of the illness and the symptoms occurred and can understand that although it was no help when they felt rejected and hurt, the changes in behaviour were in fact part of the illness.

Penelope Roques

Looking Ahead: viewpoint from Philadelphia

Picking up on one of our latest postings, I would like to remind family members that now is the time to make the inevitable mortuary arrangements, whether or not you are planning to have an autopsy. As was pointed out, no matter how long we have been living with the inevitable decline, when death does come it is still hard for all of us to accept. If the mechanics are in place, it will be just that much easier.

Another thing I would suggest was brought to my attention by one of the speakers at the Pick's support group: long term insurance. This is not for the afflicted person, but for yourself and other members of your family for whom it might be appropriate. The earlier you make your plans in this regard, the better, as the rates are very low before the age of 49 or so. You don't want your family to have to go through this financial burden again, I'm sure.

I'm not a salesman or representing any company, but it made sense to me when it was suggested to me, and as I have spent the last few months getting our finances in order (this was always handled very well by my husband, now in a nursing home) it was right for me to include my own long-term plans along with the rest.

There is nothing we can do to arrest the progress of this disease, but it sometimes helps to have something practical to turn one's attention to. I hope this helps.

Love to you all.

Tess

Please note that there is no telephone directory with this issue of the newsletter. If you need to check or find a number please refer to the old list on the Web or an old newsletter. The telephone directory is currently being updated.

Information Sheets

We have various information sheets available on:

• Pick's Disease - An Explanation
• Essential Information for Caregivers
• Recent Advances in the Genetics of Familial Alzheimer's Disease
• Lewy Body Dementia
• Primary Progressive Aphasia - What is it?
• Swallowing Problems That May Be Experienced by People Suffering From Dementia

(http://dementia.ion.ucl.ac.uk/candid/factsheets/)

Some have also been sent out with previous editions of the PDSG newsletter.

However, if you don't have access to the WWW and you or somebody you know would like more copies please send a stamped SAE to the Dementia Research Group stating clearly which one(s) you would like and we will send you them.

CONTACT DETAILS

Carol Jennings, PDSG Counsellor
8 Brooksby Close
OADBY
Leicester
LE2 5AB.
Telephone 0116 2110416
E-Mail: carol@jengs.force9.co.uk

CANDID can still be contacted on the same address and number:

Clare Morris, CANDID Counsellor
The Dementia Research Group
The National Hospital for Neurology and Neurosurgery
8-11 Queen Square
London
WC1N 3BG

Tel: 0171 829 8772
Fax: 0171 209 0182

E-mail: candid@dementia.ion.ucl.ac.uk

Penelope Roques
The Pick's Disease Support Group,
71 Myrtleside Close
Northwood
Middx
HA6 2XH
Telephone: 019238 22700
E-Mail: FrontoTemp@aol.com

PLEASE NOTE if you are receiving the newsletter by snail mail and can access it through the WWW please could you let us know your e-mail address which would save us postage.
If you have written an article, case study, or anecdote on any aspect of caring, or have any comments on the news letter, please send it to Penelope at the above address.